Thunderclap Headache Could Signal Reversible Cerebral Vasoconstriction Syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by segmental narrowing of multiple intracerebral arteries. Although this syndrome was named RCVS only recently, cases have been reported under various labels for many years. In this retrospective study, researchers report on 139 cases at Massachusetts General Hospital and Cleveland Clinic since 1993.

Patients (mean age, 42; 81% women) usually presented with severe thunderclap headaches. About half exhibited focal neurological deficits such as aphasia, hemiparesis, or ataxia, and 17% had seizures. Nine percent of cases were postpartum, and 42% of patients had been exposed to vasoconstrictive drugs. Angiography typically showed narrowing of multiple cerebral arteries bilaterally; these abnormalities reversed over time. Brain imaging abnormalities included infarction (39%), convexity subarachnoid hemorrhage (34%), and hemorrhage (20%). Most patients received either corticosteroids or calcium-channel blockers; whether these agents were beneficial is unclear. Fortunately, 78% of patients had no substantial residual disability, but 9% were left with severe deficits, and three patients died.